Flashcards in Vascular tumors Deck (17)
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1
What syndrome is associated with facial hemangioma?
PHACES
2
What anomalies are associated with PHACES syndrome (facial segmental IH)?
P- posterior fossa defects
H- hemangioma (facial segmental)
A - arterial anomalies (intracranial)
C- Cardiac anomalies (aorta)
E- eye anomalies
S- sterna clefts
3
What potentially life threatening anomaly is found with hemangioma small of the mandible?
Airway hemangioma- biphasic stridor, usually less than 6 months of age
4
Most common vascular tumors?
Infantile hemagioma
Rapidly involuting congenital hemangioma
Non involuting congenital hemangioma
Kaposiform hemangioendothelioma
Tufted angioma (angioblastoma)
5
How do you subtype infantile hemangiomas?
Distribution- Focal, multifocal, segmental
Depth- superficial, mixed, deep (below dermis)
6
Phases of IH?
Proliferation 0-12 months
Involution 1-10 yrs
Involuted
7
Pelvis Syndrome?
Perineal hemangioma
External genital malformation
Lipomyelomeningocele
Vascular anomalies
Imperforate anus
Skin tag
8
Sacral syndrome
Spinal dysraphism
Anogenital disorders
Cutaneous disorders
Renal
Associated lumbosacral hemangioma
9
Lumbar Syndrome
Lower body hemangioma
Urogenital
Myelopathy
Bony deformities
Anorectal
Arterial
Renal
10
Treatment for ulceration with IH?
Surgery
Topical Abx, hydrocoloid dressing
Steroids
Laser ablation
Propranolol
11
When do you screen for hepatic hemangioma?
- Large hemangioma in the H&N
- Lumbosacral hemangioma
- > 5 cutaneous hamngioma
- severe thrombocytopenia
12
What are the subtypes of hepatic hemangioma?
Solitary - rapidly involuting congenital hemangioma (glut-1 negative)
Mutlifocal- IH (glut-1 +) - associated cutaneous IH and heart failure
Diffuse - IH (glut-1 +) - associated hypothyroid and mortality
13
IH Theraputic options?
Observation
Drugs
Laser beams
Surgery
Drugs- propranolol, vincrisinte, INF-a
14
Congenital hemangioma types?
Rapidly involuting (usually resolved by 12-18 months)
Non-involuting (does not change with time)- requires surgery
15
Features and treatment of Kaposiform hemgioendothelioma & tufted angioma
KH - present at birth - expanding red plaque on trunk or in the mediastinum/retroperitonum
TA- present at birth- trunk/neck, dusky red plaque, may resolve or expand.
-both are associated with Kasabach-Merritt
-treatment- steroids, sirilimus, vincrisitne
16
Angiosarcoma prognostic factors?
Deep/visceral location
>5 cm
Positive margins
Local recurrence
Mets
17
